Masquerade syndrome:Retinoblastoma presenting as phacolytic glaucoma

We aim to report an uncommon presentation of retinoblastoma and its management.A 4-year-old boy presented with left eye pain for 1 week with eye redness after being hit by a ball.On examination,the visual acuity in his right eye was 6/6 and left eye was no perception to light.The conjunctiva in his left eye was injected and cornea was edematous.There was pseudohypopyon inferiorly.Lens was opaque and there was no fundus view.Intraocular pressure was 50 mmHg.Patient was initially treated as phacolytic glaucoma with severe inflammation.B scan showed intralesional calcification with retinal detachment.CT orbit showed left intraocular mass with calcification and optic nerve involvement.Thus,our final diagnosis was left eye retinoblastoma.Patient was performed a enucleation with chemotherapy in left eye.One year after the treatment,there was no local and distant invasion of the tumor.Anterior inflammation is a rare form of masquerade syndrome associated with retinoblastoma.

Retinoblastoma in an older child with secondary glaucoma as the first clinical presenting symptom: A case report

BACKGROUND Retinoblastoma(Rb)is primarily found in infants or young children.The most common primary presenting sign of Rb is leukocoria.Rb is very rare in children who are 10 years old or older.Timely and correct diagnosis as well as proper treatment are the key factors affecting the prognosis of Rb.CASE SUMMARY A 10-year-old girl with symptoms of vision loss,redness,swelling and pain in the right eye for 2 mo was admitted to our Department of Ophthalmology.The visual acuity of the right eye was graded as hand movement.The intraocular pressure of the eye was 46.9 mmHg.No substantial space-occupying lesion or characteristic calcified plaque was found in the eye.The patient underwent anterior chamber irrigation under general anesthesia on the same day of admission,and 2 mL of irrigation solution was saved for pathological examination.Histopathological examination of the anterior chamber fluid revealed cancer cells.A diagnosis of Rb with masquerade syndrome was made.The patient underwent enucleation followed by 6 rounds of systematic chemotherapy.A follow-up examination almost 9 years later found no relapse of Rb.CONCLUSION For older pediatric patients who have secondary glaucoma and uveitis symptoms without a clear cause of the disease and have no space-occupying lesion found by imaging examination,aqueous humor or vitreous humor examination is recommended for timely and correct diagnosis and appropriate treatment.

Diagnosis and management of intraocular lymphoma:a narrative review

Background and Objective:Intraocular lymphoma(IOL)is a heterogenous category of rare malignancies that are often misdiagnosed and underrecognized.The rarity of IOL impedes clinical research and contributes to difficulty in standardizing its management.In this article we review the existing scientific literature to identify the current diagnostic tools and discuss comprehensive management of various categories of IOL.Our objective is to increase disease recognition of IOL as a whole and explore updated management options for each subtype.Methods:PubMed and Embase were searched for publications using the terms‘intraocular lymphoma’,‘vitreoretinal lymphoma’,‘uveal lymphoma’,‘iris lymphoma’,‘choroidal lymphoma’and‘ciliary body lymphoma’published from 1990 to June 2021.Inclusion criteria were English language articles.Exclusion criteria were non-English language articles,case reports and animal studies.Key Content and Findings:IOL often presents in middle-aged and older patients with symptoms of floaters and vision changes,but a broad array of clinical signs and symptoms are possible depending upon subtype.IOL can be subdivided by location of involvement into vitreoretinal and uveal lymphoma.These subtypes express key differences in their pathophysiology,clinical presentation,histology,prognosis,and treatment.Primary vitreoretinal lymphomas(PVRL)generally originate from B-lymphocytes and are associated with central nervous system(CNS)lymphoma.Ophthalmic findings include retinal pigment epithelium changes with yellow subretinal deposits known as“leopard spotting.”Primary uveal lymphomas generally originate from low-grade B-lymphocytes invading the choroid and carry an improved prognosis compared to vitreoretinal lymphomas.Funduscopic findings of primary uveal lymphoma include yellow to pink-yellow choroidal swelling with infiltrative subconjunctival“salmon-patch”lesions.Diagnosis for IOL is often delayed due to insidious onset,low prevalence,and tendency to mimic diseases such as uveitis.Diagnosis may be challenging,often relying on biopsy with specialized laboratory testing for confirmation of IOL.Optimal treatment regimens are currently debated among experts.Management of IOL is best coordinated in association with neuro-oncology clinicians due to the tendency for intracranial involvement.Conclusions:IOL represents a group of multiple malignancies with distinct clinicopathologic features.Future outlook for treatment and prognosis of IOL is likely to improve with less invasive molecular diagnostic techniques and increased awareness.Clinicians should be circumspect in all patients with possible IOL and promptly refer to oncologic specialists for rapid evaluation and treatment.